MON-105 A Case Report of Neonatal Hyperinsulinism in an Infant with Turner Syndrome Successfully Treated with Diazoxide

摘要

BACKGROUND: Only four previous cases of hyperinsulinemic hypoglycemia have been reported in association with Turner Syndrome. All of these cases involved young females with a mosaic form of Turner Syndrome involving a ring X chromosome; an abnormality found in just 16% of Turner Syndrome karyotypes. Two of these cases showed responsiveness to diazoxide which stabilized their blood glucose levels and allowed for a longer fasting tolerance.